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Multifocal in situ mantle cell neoplasia of the ileocecal region: a case report with simultaneous nodal and extranodal involvement

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Abstract

In situ mantle cell neoplasia (ISMCN) is a rare entity of disputed clinical significance. We report an additional case, unusual by its presentation in the large intestine and its multifocal involvement of several nodal and extranodal sites. The diagnosis was made in a 46-year-old male patient from a surgical specimen resected for cecal adenocarcinoma. Gross examination showed multiple small polypoid lesions surrounding the ileocecal valve, corresponding to lymphoid aggregates with hyperplastic follicles. Numerous cyclin D1/SOX11+ lymphoid cells, harboring the t(11;14)(q13;q32) translocation, were present in the inner layers of mantle zones. The same lesions were found in the ileum, the appendix, and the regional lymph nodes. The final diagnosis was multifocal ISMCN of the ileocecal region, with both nodal and extra-nodal involvement. A simple surveillance was decided. Our observation expands the clinical spectrum of the disease and underlines the necessity to closely examine even normal-appearing reactive lymphoid tissues.

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Authors and Affiliations

  1. Department of Pathology, Gustave Roussy Cancer Campus, 144 rue Edouard Vaillant, 94805, Villejuif cedex, France

    Natacha Joyon, Christina Kanaan, Jean-Yves Scoazec & Peggy Dartigues

  2. Department of Tumor Genetics, Gustave Roussy Cancer Campus, Villejuif, France

    Sophie Cotteret

  3. Department of Surgery, Gustave Roussy Cancer Campus, Villejuif, France

    Isabelle Sourrouille

  4. Faculté de Médecine de Bicêtre, Université Paris Saclay, Le Kremlin-Bicêtre, France

    Jean-Yves Scoazec

Authors
  1. Natacha Joyon
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  2. Christina Kanaan
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  3. Sophie Cotteret
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  4. Isabelle Sourrouille
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  5. Jean-Yves Scoazec
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  6. Peggy Dartigues
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All authors contributed to the data acquistion, conception, and writing, and gave final approval.

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Correspondence to Natacha Joyon.

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Joyon, N., Kanaan, C., Cotteret, S. et al. Multifocal in situ mantle cell neoplasia of the ileocecal region: a case report with simultaneous nodal and extranodal involvement. Virchows Arch 479, 1037–1040 (2021). https://doi.org/10.1007/s00428-021-03065-x

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  • DOI: https://doi.org/10.1007/s00428-021-03065-x

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